She had her long-awaited EUA (exam under anesthesia) on Tuesday, April 22nd up in Chicago at UIC. We are fortunate to be under the expert care of Dr. Chan, who specializes in children's retinal disease. 

We had to be at the hospital at 5:45am, so we spent the night in Chicago. Phoebe joined us in bed around 1am, and because I was anxious for the morning appointment, I didn't get much sleep after that :P 

Thankfully, Phoebe did GREAT with all of the pre-op things... getting vitals taken, etc. which she normally fights. A chaplain came in to have a prayer for us right before, which provided such a sweet blessing of peace for us. They gave her a dose of anti-anxiety med right before taking her back to calm her down a bit, and it worked like a charm. She was wheeled away to the OR minutes later, Bluey show in hand on the anesthesiologist's phone, without a backward glance to us 😂 

They got her to sleep, dilated her eyes, and did the exam using fluorescein angiography, where they inject her with dye to light up the vessels so they can get a clear picture of what's going on . About 45 minutes in, I received a call from the Dr. telling me her left eye looks normal, but the right eye does show abnormalities consistent with what everyone has been seeing. This includes ischemia (lack of oxygen) and leakage of the abnormal vessels, which requires laser treatment. He informed that this treatment is the best course of action to close off the leaking and discourage future production of abnormal vessels. 

He performed the laser treatment, and the good news is that this is pretty uninvasive, pain-free, with little side effects. The only caveat is in rare cases, people have reported a decrease in vision or blindness in a small portion of the peripheral vision, but it's a case where the benefits outweigh that risk. 

The procedure was successful, and she was soon in recovery. She woke up pretty hard-- very tired, loopy, angry, irritable. She wanted nothing to do with anything... no one touching her, offering popsicles, etc. Poor girl! We were actually able to leave pretty quickly, and were back to our car by 11am to head home. She went right to sleep in the car, slept for 2 hours, then woke up and started snacking on everything in sight. She was hungry!

She was totally great the rest of the day, and acted as if nothing had happened to her that morning. It was so reassuring to see her acting perky and happy. 

We will follow up with Dr. Chan the beginning of June to make sure the laser treatment is doing its job. He suspects we may need another EUA in a year to keep an eye on the disease.

The retinal disease she has is called FEVR (Familial Exudative Vitreoretinopathy.) It's a rare genetic disease that affects how the vessels develop in the retina. Symptoms can range from mild (some may have this condition and never know it) to severe, where retinal folding, leaking, bleeding, vision loss, and retinal detachment can take place.

While she was in her EUA, they did collect blood to genetically test for FEVR, but the results may not be the best diagnostic tool. Only 50-60% of cases with FEVR test positive for it, likely because there are undiscovered genetic variants that wouldn't be tested. So while getting a positive result would definitely give us confirmation of the disease, getting a negative result doesn't rule it out, and after her recent exam, it's the very likely cause.

We are pretty certain this genetic disease comes from my side of the family :) as eye issues can be traced back pretty far on both sides of my family. I was born with crossed eyes, had a lazy eye, and required two eye surgeries to correct my crossed eyes. My oldest sister also had crossed eyes. This can definitely be a symptom of a mild form of FEVR, where as Phoebe's abnormal blood vessels is the hallmark manifestation of the disease. 

We may possibly need to test Ryan & I for FEVR to understand where the disease was passed from, as well as our kids. The main thing for now is to keep a close watch on Phoebe via her ophthalmologist, as this disease is most active in childhood and can progress quietly left unchecked. The hope is to get her to a stable place, in which case she should be able to lead a relatively normal outlook.

Possible future issues could be a lazy eye (which is actually what prompted us to check in the first place, as the affected eye does pull in a bit)-- where the brain favors the stronger eye, depth perception issues, or at worst, vision loss.

While I'm not thrilled about this diagnosis for Phoebe, I find that my perspective is colored by my own experience. Having grown up with limitations on my own eyes, I know it's not a death sentence. To this day, I struggle with a left-dominant eye-- because my right eye was "lazy" as a child, my brain learned to favor my left eye, and it does most of the work. If I lost vision in my right eye completely, I honestly don't think I'd notice a huge difference. Because of this, I have depth perception issues and have never been super coordinated with sports. I also have very poor vision, with my prescription being at -8.5 and -9.0, and am nearly blind without glasses or contacts in. 

Interestingly enough, I ended up in a visual field! As far as I know, this has not affected my ability to be a photographer, "see" creatively, and enjoy the art of life. Because of this, I know that whatever Phoebe has to deal with regarding this disease, she will be OK. And she has a Mom that understands. :)

"The Lord is your shade at your right hand; the sun will not harm you by day, nor the moon by night. The Lord will keep you from all harm; he will watch over your life. The Lord will watch over your coming and going both now and forevermore."  -Psalm 121:5-8